Clinical practice guidelines for the management of atypical haemolytic uraemic syndrome in the United Kingdom

Br J Haematol. 2010 Jan;148(1):37-47. doi: 10.1111/j.1365-2141.2009.07916.x. Epub 2009 Oct 11.


Atypical haemolytic uraemic syndrome (aHUS) is associated with a poor prognosis with regard to survival at presentation, recovery of renal function and transplantation. It is now established that aHUS is a disease of complement dysregulation with mutations in the genes encoding both complement regulators and activators, and autoantibodies against the complement regulator factor H. Identification of the underlying molecular abnormality in an individual patient can now help to guide their future management. In these guidelines we make recommendations for the investigation and management of aHUS patients both at presentation and in the long-term. We particularly address the role of renal transplantation alone and combined liver-kidney transplantation.

Publication types

  • Practice Guideline

MeSH terms

  • Antibodies, Monoclonal / therapeutic use
  • Antibodies, Monoclonal, Humanized
  • Clinical Trials as Topic
  • Hemolytic-Uremic Syndrome / classification
  • Hemolytic-Uremic Syndrome / diagnosis
  • Hemolytic-Uremic Syndrome / therapy*
  • Humans
  • Kidney Transplantation
  • Liver Transplantation
  • Plasma Exchange / methods
  • Terminology as Topic


  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Humanized
  • eculizumab